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Management of cervicofacial lymphatic malformations requires a multidisciplinary approach.

Authors :
Zobel, Michael J.
Nowicki, Donna
Gomez, Gabriel
Lee, Jessica
Howell, Lori
Miller, Joseph
Zeinati, Chadi
Anselmo, Dean M.
Source :
Journal of Pediatric Surgery; May2021, Vol. 56 Issue 5, p1062-1067, 6p
Publication Year :
2021

Abstract

Cervicofacial lymphatic malformations (CFLM) are rare, potentially life-threatening vascular anomalies, yet reports on multidisciplinary treatment strategies are lacking. We evaluated outcomes for CFLMs following sclerotherapy, surgical resection, and/or medical management. We identified children with a CFLM at a vascular anomalies center from 2004 to 2019. Exclusion criteria: retro-orbital malformations, untreated malformations, patients without follow-up. Primary clinical outcome was contour improvement, with significance defined as LM volume reduction of > 50% by cross-sectional imaging. Sixty-three children met inclusion criteria: 35 with macrocystic CFLMs, six with microcystic CFLMs, and 22 with mixed-type malformations. Mean post-intervention follow-up was 27.5 months. Fifty-eight patients underwent sclerotherapy (median: two treatments). Doxycycline and/or bleomycin were used in 95% of patients. After sclerotherapy, 97% of macrocystic CFLMs improved significantly compared to 82% of mixed and 67% of microcystic lesions. Sixteen children underwent surgical resection with 75% significantly improving; two additional patients were successfully treated with sclerotherapy after debulking surgery. Six children received sirolimus for microcystic disease, of which 33% significantly improved. Sclerotherapy is very effective for macrocystic components of CFLMs, albeit less so for microcystic disease. Microcystic CFLMs frequently require surgical resection. Sirolimus is a helpful therapeutic adjunct, particularly for microcystic lesions, but more study is needed. Level II, prognosis study [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00223468
Volume :
56
Issue :
5
Database :
Supplemental Index
Journal :
Journal of Pediatric Surgery
Publication Type :
Academic Journal
Accession number :
150207229
Full Text :
https://doi.org/10.1016/j.jpedsurg.2020.09.017